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Query: UNIPROT:P02794 (ferritin)
 17,525 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Recurrences of CNS infarction often lead to progressive neurologic disability in sickle cell anemia. To prevent such reccurrence, a periodic blood transfusion program was begun in 1969. Currently, 27 patients are on this regimen. Before inclusion in the program, 12 patients had had one to nine CNS recurrences each. Since the program was started, two patients have had transient CNS ischemia. There were no other recurrences and none of the patients have shown progression of neurologic abnormalities. In addition, there was a striking decrease in bacterial infection and pain. We conclude that periodic transfusions are effective in preventing recurrent CNS infarction in sickle cell anemia. The benefits must be weighed against the potentially serious problem of iron overload, as evidenced by moderately elevated serum ferritin values.
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PMID:Periodic transfusions for sickle cell anemia and CNS infarction. 51 76

Total body iron burden was estimated by two indirect methods in 23 patients with sickle cell anemia. Concentrations of serum ferritin correlated directly and significantly with age of the patients. Eleven of 15 patients under 20 years of age had normal levels of serum ferritin. Deferoxamine-induced urinary excretion of iron was considerably less than that reported in patients with thalassemia major who were receiving regular blood transfusions. These data imply that patients with sickle cell anemia generally do not acquire excessive iron burdens during the first two decades of life. The risks of transfusional hemosiderosis in patients with sickle cell anemia who are included in hypertransfusion programs are discussed.
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PMID:Iron burden in sickle cell anemia. 63 16

In a group of 35 children with sickle cell anaemia serum ferritin concentration ranged from 70 to 2460 microgram/l (mean 367, median 180 microgram/l). This was significantly higher than the ferritin levels (range 8-101, mean 34, median 30 microgram/l) in a group of 63 normal control children of the same age group. 30 (86%) of the sickle cell children showed serum ferritin levels greater than 101 microgram/l, and 2 (6%) levels greater than 1000 microgram/l. 7 of the patients had not been transfused before this study. Their serum ferritin levels were all raised and showed a significant correlation with age but not with haemoglobin level. In the remainder of the patients the serum ferritin bore no significant correlation with age, haemoglobin level, or number of units of blood transfused. 2 children with HbSC disease had levels within the control range. Since patients with sickle cell anaemia have an increasing chance of long survival, we suggest that serial estimations of their iron status be made by means of serum ferritin assay in order to determine which patients are accumulating excessive iron.
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PMID:Value of serum ferritin estimation in sickle cell anaemia. 64 43

In an attempt to understand the variability of the hematologic response to oral sodium cyanate, iron metabolism was studied in a group of 39 patients with sickel cell disease. Eleven of the 39 patients were found to have no stainable iron in the marrow despite the fact that patients with sickle cell disease are generally considered to have hemosiderosis. The mean per cent saturation and total iron-binding capacity were in the low-normal range in sickle cell patients whether or not stainable iron was present in the bone marrow aspirate. Serum ferritin concentrations, on the other hand, were found to be high in both groups (greater than 500 mu g/liter) when compared to controls (60 mu g/liter). The high serum ferritin levels denoted significant total-body iron deposition which may be unavailable for normal metabolic processes. One patient with no stainable iron in the bone marrow aspirate did respond to iron therapy alone with an increase in hemoglobin concentration. Serum ceruloplasmin levels were also found to be high in sickle cell disease patients. The ability to respond to oral cyanate therapy was correlated with the amount of stainable iron in the bone marrow aspirate. These studies emphasize the necessity of a reevaluation of iron metabolism in the pathophysiology and treatment of sickle cell disease.
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PMID:Iron metabolism, sickle cell disease, and response to cyanate. 117 92

The nutritional status and dietary intake of children with sickle cell anemia were examined to confirm the presence of deficiencies. Nine children with sickle cell anemia and 19 controls were assessed. Resting energy expenditure was measured with open circuit indirect calorimetry and body composition was estimated from skinfold measurements. Three-day food records were analyzed for protein, zinc, vitamin A, folic acid, and iron content. Serum vitamin A, red blood cell zinc, red blood cell folate, serum folate, serum ferritin, hematocrit, total urine nitrogen, and 24-h urine creatinine levels were measured. The children with sickle cell anemia were leaner, weighed less, and had lower red blood cell zinc levels, lower serum vitamin A levels, lower urine nitrogen levels, and greater resting energy expenditure than controls. Serum and red blood cell folate levels were within normal ranges and similar to controls. The serum ferritin level was higher than controls and higher than normal for age. Dietary intake of energy and protein was more than that of controls, and dietary intake of zinc, vitamin A, folic acid, and iron was similar to that of controls and adequate compared to the Recommended Dietary Allowances. Further studies are needed to explain the physical and biochemical differences seen in children with sickle cell anemia despite dietary intakes that appear to be adequate and similar to those of controls.
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PMID:Nutritional status and dietary intake of children with sickle cell anemia. 155 Feb 64

In evaluating pregnant women with anemia, it is essential to do a complete history and physical examination, as well as a complete blood count with indices and a blood smear examination. Based on these findings, other tests such as ferritin and serum or red cell folate may be ordered. Because of the normal physiologic changes in pregnancy that affect the hematocrit, indices, and some other parameters, diagnosing true anemia, as well as the etiology of anemia, is challenging. Because of the increased nutritional requirements of the mother and fetus, the most common anemias are iron deficiency anemia and folate deficiency megaloblastic anemia. These anemias are more common in women who have inadequate diets and who are not receiving prenatal iron and folate supplements. Other less common causes of acquired anemia in pregnancy are aplastic anemia and hemolytic anemia associated with preeclampsia. In addition, congenital anemias such as sickle cell disease can impact on the health of the mother and fetus. Obviously, severe anemia has adverse effects on the mother and the fetus. There is also evidence that less severe anemia is associated with poor pregnancy outcome. The cause of this association has yet to be elucidated. It is important, however, to diagnose and treat anemia in pregnancy to provide for optimal health of the mother and infant.
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PMID:Anemia in pregnancy. 157 61

Persons with sickle cell anemia have several indications for transfusion of red blood cells. One of the complications of transfusion of red blood cells is iron overload. Iron overload has been associated with multiple endocrine abnormalities. We report herein three cases of hypothyroidism in adult individuals with sickle cell disease. All three patients were over the age of 45 years at the time of the diagnosis and had received multiple units of transfused red blood cells and had serum ferritin levels of greater than 6,000 ng/mL. All patients were diagnosed during times when they were critically ill. Replacement therapy was instituted in all cases; however, all three patients died shortly after the diagnosis of hypothyroidism was made. Congestive heart failure appeared to be a primary cause of death in all three patients. In the one patient in whom a postmortem examination was done, there was evident extensive fibrosis of the thyroid gland as well as extensive deposition of iron in the cells lining the thyroid follicles. We believe that this represents the first report of clinical hypothyroidism in patients with sickle cell anemia who have received multiple transfusions. Awareness of this condition is especially important given that congestive heart failure is common in sickle cell disease.
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PMID:Hypothyroidism in adults with sickle cell anemia. 158 Mar 5

Iron deficiency anaemia was detected in 23% of cases with homozygous sickle cell disease. The aetiology of iron deficiency was similar to the other population in the community. High serum ferritin level was detected in 15.4% of the cases and was well correlated to the number of transfusions. Tissue haemosiderosis was not detected in any case. Patients with heterozygous sickle cell had either normal or low serum ferritin levels.
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PMID:Iron in sickle cell disease. 146 1

Because peroxidative damage to red cell membranes may contribute to the pathophysiology of sickle cell disease, deficiency of fat- and water-soluble antioxidants could be a determinant in the pathogenesis of this disease. We have previously reported a deficiency of vitamin E in sickle cell disease. The present study was undertaken to see if a deficiency in vitamin C might also be detected. Leukocyte vitamin C, which reflects total body vitamin C reserve, was measured by a modified 2,4-dinitrophenylhydrazine method. Sickle cell patients (N = 18) had lower leukocyte vitamin C levels (18.3 +/- 9.4 micrograms/10(8) cells) than normal controls (N = 12; 30.3 +/- 7.5 micrograms/10(8) cells; p less than 0.01). Furthermore, 50% of the patients had vitamin C levels below 15 micrograms/10(8) cells, a value consistent with vitamin C deficiency. A statistically significant correlation (r = -0.62 with 0.01 less than p less than or equal to 0.025) was found between leukocyte vitamin C levels and serum ferritin concentration. Because dietary vitamin C intake appeared to be adequate, increased vitamin C utilization may account for this deficiency. However, the mechanisms for this deficiency as well as its pathophysiologic consequences remain to be established.
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PMID:Vitamin C deficiency in patients with sickle cell anemia. 224 Apr 72

37Fe Moessbauer spectroscopy has been applied to the study of iron deposits in patients with altered iron metabolism. Haematological parameters were also studied in order to analyse their relationship with Moessbauer results. Within the aim of this research, 12 samples of packed red blood cells were analysed: 6 with beta-thalassaemia major, 2 with S-beta-thalassaemia, 1 with sickle cell anaemia and 3 from normal subjects used as control for Moessbauer spectroscopy. Moessbauer spectra of 6 red blood cells samples showed that besides the two components, i.e., oxy and deoxy haemoglobin present in samples of normal subjects, appears a third component with Moessbauer parameters corresponding to ferritin-like iron. Correlation of % transferrin saturation (TS %) with ferritin-like iron (r = 0.90, p less than 0.05) as well as between TS % and the ratio ferritin-like iron/Hb iron (r = 0.91, p less than 0.05) was found. A tendency to correlation of serum ferritin (SF) with ferritin-like iron (r = 0.90, p less than 0.05) as well as between TS % and the ratio ferritin-like iron/Hb iron (r = 0.91, p less than 0.05) was found. A tendency to correlation of SF with ferritin-like iron (r = 0.78) and with the ratio ferritin-like iron/Hb iron, was also observed. It can be concluded that Moessbauer spectroscopy could be a useful technique in the study of this kind of pathology.
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PMID:[Application of Mossbauer spectroscopy to the study of hemoglobinopathies. Preliminary experience]. 261 81


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